Overview of Long-Term Outcome in Adults with Systemic Right Ventricle and Transposition of the Great Arteries: A Review

Abstract

The population of patients with a systemic right ventricle (sRV) in biventricular circulation includes those who have undergone an atrial switch operation for destro-transposition of the great arteries (d-TGA) and those with congenitally corrected transposition of the great arteries (ccTGA). Despite the life expectancy of these patients is significantly increased, the long-term prognosis remains suboptimal due to late complications such as heart failure, arrhythmias, and premature death. These patients, therefore, need a close follow-up to early identify predictive factors of adverse outcomes and to implement all preventive therapeutic strategies. This review analyzes the late complications of adult patients with an sRV and TGA and clarifies which are risk factors for adverse prognosis and which are the therapeutic strategies that improve the long-term outcomes. For prognostic purposes, it is necessary to monitor sRV size and function, the tricuspid valve regurgitation, the functional class, the occurrence of syncope, the QRS duration, N-terminal pro B-type natriuretic peptide levels, and the development of arrhythmias. Furthermore, pregnancy should be discouraged in women with risk factors. Tricuspid valve replacement/repair, biventricular pacing, and implantable cardioverter defibrillator are the most important therapeutic strategies that have been shown, when used correctly, to improve long-term outcomes.

Transcatheter edge-to-edge repair of both atrioventricular valves in congenitally corrected transposition of the great arteries: a case report

“… 6 This is especially important, as the natural history and clinical presentation of ccTGA patients is determined by associated malformations. 1 , 2 Even in the case of isolated ccTGA, only around 50% of patients reach the age of 60. 1 , 7 Current guidelines 7 , 8 recommend typical HFrEF medical therapy and palliative TV replacement in selected cases, as corrective surgery, e.g.…”

“…Furthermore, ccTGA patients are often discovered at advanced ages or with signs of advanced systemic ventricle failure and are most frequently deemed inoperable. 2 In this setting, transcatheter treatment of the systemic tricuspid valve (TV) by transcatheter edge-to-edge repair (TEER) is gaining ground. 3 However, to date, reports on TEER treatment of both AV valves are lacking.…”

Challenges and opportunities in patients with adult congenital heart disease, a narrative review

“…In individuals presenting with a systemic morphologic RV, the likelihood of HF progression over time is estimated to be present in 65% of these patients by the age of 45 years of age (64)(65)(66). Our institution adopts a cautious approach when considering the administration of conventional left HF medications to this specific patient group.…”